Atypical Teratoid Rhabdoid Tumor

Atypical teratoid rhabdoid tumor (AT/RT) is a rare tumor usually diagnosed in childhood. Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS) including the spinal cord. About 60% will be in the posterior cranial fossa (particularly the cerebellum). One review estimated 52% posterior fossa, 39% sPNET (supratentorial primitive neuroectodermal tumors), 5% pineal, 2% spinal, and 2% multi-focal.

In the United States, three children per 1,000,000 or around 30 new AT/RT cases are diagnosed each year. AT/RT represents around 3% of pediatric cancers of the CNS. Around 17% of all pediatric cancers involve the CNS; it is the most common childhood solid tumor. The survival rate for CNS tumors is around 60%; with AT/RT it is around 10%. Pediatric brain cancer is the second leading cause of childhood death, just after leukemia. Recent trends suggest that the rate of overall CNS tumor diagnosis is increasing by about 2.7% per year. As diagnostic techniques using genetic markers improve and are used more often, the proportion of AT/RT diagnoses is expected to increase.

Because it is highly malignant, AT/RT has a high mortality rate. A survey of 36 AT/RT patients at St. Jude Children's Research Hospital from 1984 to 2003 showed that the two-year event-free survival for children under three was 11%, the overall survival rate was 17%. For children aged 3 years or older the event-free survival was 78% and the overall survival 89%. Because most patients with AT/RT are less than three years old (71% in St Jude Study), the overall prognosis for AT/RT is very poor.

Current research is focusing on using chemotherapy protocols that are effective against rhabdomyosarcoma in combination with surgery and radiation therapy.

Read more about Atypical Teratoid Rhabdoid Tumor:  Classification, Signs and Symptoms, Genetics, Pathology, Diagnosis, Prognosis, Epidemiology, History, Research Directions, Cultural References

Other articles related to "atypical teratoid rhabdoid tumor, atypical teratoid, tumor, rhabdoid tumor":

Atypical Teratoid Rhabdoid Tumor - History
... Atypical teratoid/rhabdoid tumor was first described as a distinct entity in 1987 ... Before 1978, when rhabdoid tumor was described, AT/RT likely was misdiagnosed as medulloblastoma ... In some early reports the tumor was known also as malignant rhabdoid tumor (MRT) of the CNS ...
Atypical Teratoid Rhabdoid Tumor - Cultural References
... In August 2011, a six-year-old named Avalanna Routh who was battling AT/RT at Dana–Farber Cancer Institute was given a pretend wedding with her idol Justin Bieber, with doctors and nurses providing a cardboard life-sized cutout of Bieber, a guitarist, flowers, and a T-shirt emblazoned with the words "Future Mrs ... Bieber" ...